Natural history and functional status of patients with myotubular myopathy enrolled in a prospective and longitudinal study

2015 ◽  
Vol 25 ◽  
pp. S274
Author(s):  
N. R'guiba ◽  
M. Annoussamy ◽  
R. Cardas ◽  
C. Lilien ◽  
G. Ollivier ◽  
...  
2010 ◽  
Vol 37 (2) ◽  
pp. 280-288 ◽  
Author(s):  
J. M. Habraken ◽  
W. M. van der Wal ◽  
G. ter Riet ◽  
E. J. M. Weersink ◽  
F. Toben ◽  
...  

2016 ◽  
Vol 25 (2) ◽  
pp. 144-151 ◽  
Author(s):  
Fidel López-Espuela ◽  
Juan Diego Pedrera-Zamorano ◽  
Pedro Enrique Jiménez-Caballero ◽  
José María Ramírez-Moreno ◽  
Juan Carlos Portilla-Cuenca ◽  
...  

Background Stroke is a major public health problem. Objective To use the Barthel Index to evaluate basic activities of daily living in stroke survivors and detect any predictors of functional outcome at 6 months after stroke. Methods In an observational longitudinal study, data were gathered on consecutive patients admitted to the comprehensive stroke unit at Hospital San Pedro de Alcantara, Cáceres, Spain. Sociodemographic and clinical data were obtained prospectively at hospital admission and during follow-up 6 months later. Information on type of stroke, score on the Barthel Index, findings from the neurological evaluation, and other relevant data were collected. Results Of 236 patients admitted, 175 participated in the study. Mean age was 69.60 (SD, 12.52) years, 64.6% were men, and mortality was 12.8%. Six months after experiencing a stroke, 84.8% of patients had returned to their own homes, 8.0% were institutionalized, and the others were residing at a family member’s home. Scores on the Barthel Index 6 months after stroke correlated with baseline scores on the National Institute of Health Stroke Scale (r = −0.424; P < .001) and with depressive mood 6 months after stroke (r = −0.318; P < .001). Age was negatively associated with Barthel Index scores at the time of hospital discharge and 6 months after stroke. Conclusions Functional status 6 months after stroke was influenced by age, sex, stroke severity, type of stroke, baseline status, mood, and social risk. Comorbid conditions, socioeconomic level, and area of residence did not affect patients’ functional status.


2020 ◽  
Author(s):  
Lizhi Guo ◽  
Li An ◽  
Fengping Luo ◽  
Bin Yu

Abstract Objective This study investigated whether loneliness or social isolation is associated with the onset of functional disability over 4 years among Chinese older populations. Setting and Subjects This study used data from the China Health and Retirement Longitudinal Study (CHARLS). Functional status was assessed by activities of daily living (ADL) and instrumental activities of daily living (IADL). Analyses were conducted with data from two waves (2011 and 2015) and were restricted to those respondents aged 50 and older and free of functional disability at baseline [n = 5,154, mean age (SD) = 60.72 (7.51); male, 52.3%]. Method Social isolation, loneliness and covariates were measured at baseline. Follow-up measures of new-onset ADL and IADL disability were obtained 4 years later. We stratified the sample by gender, and then used binary logistic regressions to evaluate the associations between baseline isolation, loneliness and new-onset ADL and IADL disability. Results For women, baseline social isolation was significantly associated with new-onset ADL (OR = 1.18, 95% CI = 1.07–1.30) and IADL (OR = 1.11, 95% CI = 1.01–1.21) disability; no significant association between loneliness and ADL or IADL disability was found. For men, neither social isolation nor loneliness was found to be significantly associated with ADL or IADL disability. Conclusion This longitudinal study found that social isolation, rather than loneliness, was significantly associated with functional disability over 4 years among women (but not men) in China. These findings expand our knowledge about the association between social relationships and functional status among non-Western populations.


2008 ◽  
Vol 85 (3) ◽  
pp. 818-821 ◽  
Author(s):  
Alessandro Giardini ◽  
Alfred Hager ◽  
Carlo Pace Napoleone ◽  
Fernando M. Picchio

BMJ Open ◽  
2019 ◽  
Vol 9 (6) ◽  
pp. e026286 ◽  
Author(s):  
Amaria Baghdadli ◽  
Stéphanie Miot ◽  
Cécile Rattaz ◽  
Tasnime Akbaraly ◽  
Marie-Maude Geoffray ◽  
...  

IntroductionThere is global concern about the increasing prevalence of autism spectrum disorders (ASDs), which are early-onset and long-lasting disorders. Although ASDs are considered to comprise a unique syndrome, their clinical presentation and outcome vary widely. Large-scale and long-term cohort studies of well-phenotyped samples are needed to better understand the course of ASDs and their determinants. The primary objective of the multicEntric Longitudinal study of childrEN with ASD (ELENA) study is to understand the natural history of ASD in children and identify the risk and prognostic factors that affect their health and development.Methods and analysisThis is a multicentric, longitudinal, prospective, observational cohort in which 1000 children with ASD diagnosed between 2 and 16 years of age will be recruited by 2020 and followed over 6 years. The baseline follow-up starts with the clinical examination to establish the ASD diagnosis. A battery of clinical tools consisting of the Autism Diagnostic Observation Schedule, the revised version of the Autism Diagnostic Interview, measures of intellectual functioning, as well as large-scale behavioural and developmental measurements will allow us to study the heterogeneity of the clinical presentation of ASD subtypes. Subsequent follow-up at 18 months and at 3, 4.5 and 6 years after the baseline examination will allow us to explore the developmental trajectories and variables associated with the severity of ASD. In addition to the children’s clinical and developmental examinations, parents are invited to complete self-reported questionnaires concerning perinatal and early postnatal history, congenital anomalies, genetic factors, lifestyle factors, medical and psychiatric comorbidities, and the socioeconomic environment. As of 1 November 2018, a total of 766 participants have been included.Ethics and disseminationEthical approval was obtained through the Marseille Mediterranean Ethics Committee (ID RCB: 2014-A01423-44), France. We aim to disseminate the findings through national and international conferences, international peer-reviewed journals, and social media.Trial registration numberNCT02625116; Pre-results.


2019 ◽  
Vol 31 (12) ◽  
pp. 1709-1719 ◽  
Author(s):  
Qinqin Meng ◽  
Huali Wang ◽  
John Strauss ◽  
Kenneth M. Langa ◽  
Xinxin Chen ◽  
...  

ABSTRACTObjective:To compare and validate neurocognitive tests in the Harmonized Cognitive Assessment Protocol (HCAP) for the China Health and Retirement Longitudinal Study (CHARLS), and to identify appropriate tests to be administered in future waves of CHARLS.Methods:We recruited 825 individuals from the CHARLS sample and 766 subjects from hospitals in six provinces and cities in China. All participants were administered the HCAP-neurocognitive tests, and their informants were interviewed regarding the respondents’ functional status. Trained clinicians administered the Clinical Dementia Rating scale (CDR) to assess the respondents’ cognitive status independently.Results:The testing protocol took an average of 58 minutes to complete. Refusal rates for tests of general cognition, episodic memory, and language were less than 10%. All neurocognitive test scores significantly correlated with the CDR global score (correlation coefficients ranged from 0.139 to 0.641). The Mini-Mental State Examination (MMSE), the Health and Retirement Study (HRS) - telephone interview for cognitive status (TICS), community screening instrument for dementia (CSI-D) for respondent, episodic memory and language tests each accounted for more than 20% of the variance in global CDR score (p < 0.001) in bivariate tests. In the CHARLS subsample, age and education were associated with neuropsychological performance across most cognitive domains, and with functional status.Conclusion:A brief set of the CHARLS-HCAP neurocognitive tests are feasible and valid to be used in the CHARLS sample and hospital samples. It could be applied in the future waves of the CHARLS study, and it allows estimating the prevalence of dementia in China through the population-based CHARLS.


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